A challenging case report of IgG4‐related systemic disease involving the heart and retroperitoneum with a literature review of similar heart lesions

• Published in: Echocardiography (Mount Kisco, N.Y.) Vol. 37; no. 9; pp. 1478 - 1484
• Main Authors: Hajsadeghi, Shokoufeh, Pakbaz, Marziyeh, Hassanzadeh, Morteza, Sadeghipour, Alireza
• Format: Journal Article
• Language: English
• Published: United States 01-09-2020
• Subjects: IgG4‐related disease; pericardial effusion; pituitary gland; retroperitoneum; pericardial effusion; IgG4-related disease; pituitary gland; retroperitoneum
• ISSN: 0742-2822; 1540-8175
• DOI: 10.1111/echo.14828

The IgG4‐related disease is a distinct, steroid‐responsive fibro‐inflammatory disorder of unknown etiology. This multiorgan disease is characterized by tumefactive lesions that contain rich infiltrations of IgG4‐positive plasma cells, with the pancreas, and the salivary and lacrimal glands being the main involved. The more common cardiovascular involvements include inflammatory peri‐aortitis, coronary arteritis, and pericarditis. Intra‐cardiac tumefactive lesions are rarely reported. Herein, we describe a challenging case of IgG4‐related disease with a long‐time lag between initiation of symptoms to proper diagnosis with biopsy‐proven cardiac and retroperitoneal and possible pituitary gland involvement. Concerning the rarity of the cardiac lesion in our case, we conducted a literature review of similar case reports.